Severe pulmonary hypertension in liver transplant candidates

Advanced liver disease with portal hypertension may be associated with pulmonary hypertension. A review of 1,205 consecutive liver transplant patients was made to assess the incidence and severity of pulmonary hypertension in patients with end-stage liver disease. Postoperative data were reviewed to determine if outcome was influenced and, in patients with severe pulmonary hypertension, whether pulmonary hypertension was reversed after transplantation. The hemodynamic data of 5 patients who were found to have severe pulmonary hypertension before transplantation and did not receive transplants were also reviewed. The incidence of pulmonary hypertension in the patients who received transplants was 8.5% (n = 102; mean pulmonary artery pressure, > 25 mmHg). The incidence of mild pulmonary hypertension was 6.72% (n = 81; systolic pulmonary artery pressure, 30 to 44 mmHg); that of moderate pulmonary hypertension was 1.16% (n = 14; systolic pulmonary artery pressure, 45 to 59 mmHg); and that of severe pulmonary hypertension was 0.58% (n = 7; systolic pulmonary artery pressure, > 60 mmHg). Mild and moderate pulmonary hypertension did not influence the outcome of the procedure. Severe pulmonary hypertension was associated with mortality rates of 42% at 9 months posttransplantation and 71% at 36 months posttransplantation. Only 2 of 7 patients with severe pulmonary hypertension have survived liver transplantation with a good quality of life. The remaining 5 patients continued to deteriorate with progressive right heart failure with no evidence of amelioration of the pulmonary hypertension. This experience supports the view that in most patients who have severe pulmonary hypertension associated with advanced liver disease, it is caused by fixed pathological changes in the pulmonary vasculature, is not reversible with liver transplantation, and is associated with a very high perioperative mortality rate. (Liver Transpl Surg 1997 Sep;3(5):494-500)     

Inhaled nitric oxide in the preoperative evaluation of pulmonary hypertension in heart transplant candidates

OBJECTIVE: The goal of this study was to evaluate the efficacy of 100% oxygen and inhaled nitric oxide (iNO) in decreasing pulmonary vascular resistance (PVR) and transpulmonary gradient (TPG) in dilated cardiomyopathy patients being evaluated for orthotopic heart transplantation (OHT); who, despite maximal intravenous (IV) dilator therapy, had persistent moderate-to-severe pulmonary hypertension. DESIGN: A prospective nonrandomized clinical study. SETTING: University hospital, major transplant center. PARTICIPANTS: Twenty-one adult patients undergoing OHT evaluation. Interventions: One hundred percent oxygen and iNO at 20 and 40 ppm were sequentially administered to the patients once they were optimized with IV vasodilators and inotropes. MEASUREMENTS AND MAIN RESULTS: Although no significant change was noted with oxygen, iNO 20 ppm reduced the mean pulmonary artery pressure (44.1 +/- 1.7 to 38.6 +/- 1.8 mmHg, p < 0.05), PVR index (823 +/- 47 to 621 +/- 55 dyne/s/m(2)/cm(5), p < 0.05), TPG (22.4 +/- 1.4 to 17.0 +/- 1.5 mmHg, p < 0.05), and right ventricular stroke work index (14.7 +/- 1.2 to 11.1 +/- 1.2 g . m/m(2)/beat, p < 0.05). In 13 of 21 patients, PVR decreased by greater than 25% after iNO therapy. Nine of these patients had PVR and TPG decrease to levels considered acceptable for OHT listing. CONCLUSIONS: iNO can further improve right ventricular hemodynamics even after presumed optimization with IV vasodilators and serves as a test of PVR reversibility during the preoperative assessment of OHT candidates.     

Effectiveness and safety of treatment with sildenafil for secondary pulmonary hypertension in heart transplant candidates

BACKGROUND: Pulmonary hypertension (PH) is a predictor of early death risk owing to right heart insufficiency after orthotopic heart transplantation (OHT). The aim of this study was to evaluate the effectiveness and safety of sildenafil therapy to decrease pulmonary vascular resistance (PVR) in patients with heart failure requiring transplantation, who may otherwise have been excluded because of PH. MATERIAL AND METHODS: We analyzed the hemodynamic results of six men (aged 47 to 61) with well-grounded OHT indications and PH diagnosed by a transpulmonary gradient (TPG) > 12 mmHg and/or PVR > 2.5 Wood units. Patients underwent a PH reversibility test with sodium nitroprusside (NPS) to achieve normal TPG and PVR results without a drop in systolic arterial pressure <85 mmHg. Unresponsiveness to NPS was shown in all subjects, who were subsequently qualified for sildenafil therapy (50 mg bid). RESULTS: After 1 month of sildenafil, three subjects achieved normal TPG and PVR, and acceptable responsiveness of PH to NPS in two other patients, all of whom qualified for OHT. Therapy was unsuccessful in one patient, which was confirmed also by right heart catheterization after 3 months of sildenafil use. Therapy was well tolerated in all patients, namely, no significant drop in arterial pressure on angiotensin-converting enzyme inhibitors. CONCLUSIONS: Sildenafil may be effectively used for treatment of secondary, irreversible PH in potential heart transplant recipients.     

Reversible catecholamine-induced cardiomyopathy in a heart transplant candidate without persistent or paroxysmal hypertension.

BACKGROUND: Both dilated and hypertrophic cardiomyopathy have been reported in patients with pheochromocytoma, who were almost always hypertensive. The outcome frequently has been fatal, yet cardiac dysfunction can be reversible after medical or surgical therapy for the pheochromocytoma. METHODS: We report the case of a patient with dilated cardiomyopathy without persistent or paroxysmal hypertension, who was found to have a pheochromocytoma during initial medical evaluation. RESULTS: The identification and treatment of the pheochromocytoma led to significant improvement in cardiac function and cardiac transplantation was avoided. CONCLUSIONS: This case illustrates some unusual features in pheochromocytoma-induced cardiomyopathy: (1) absence of persistent or paroxysmal hypertension, (2) initial presentation with acute myocardial infarction and normal coronary arteries, and (3) recurrent episodes of nonsustained ventricular tachycardia.     

Selective pulmonary vasodilation with inhaled aerosolized milrinone in heart transplant candidates

PURPOSE: Selective pulmonary vasodilation is an advantageous method for testing the responsiveness of the pulmonary vasculature of heart transplant candidates. A pilot study was under-taken to test the hypothesis that inhaled aerosolized milrinone may cause selective pulmonary vasodilation. METHODS: 18 consecutive male heart transplant candidates with either dilated or ischemic cardiomyopathy were included in this open clinical study. Nine of the patients had significant pulmonary hypertension with a mean pulmonary arterial pressure > 30 mmHg. After baseline measurements, 2 mg of milrinone was administered by ultrasonic nebulization. Pulmonary and systemic hemodynamics were measured ten, 30, and 60 min after inhalation. RESULTS: After inhalation for ten minutes, milrinone induced a significant reduction of mean pulmonary arterial pressure (32.7 +/- 9.1 vs 37.7 +/- 7.5 mmHg, P = 0.01), pulmonary vascular resistance index (296 +/- 150 vs 396 +/- 151 dyn.sec(-1).cm(-5).m(2), P = 0.02) and transpulmonary gradient (10.6 +/- 5.5 vs 15 +/- 4.9, P = 0.01) only in patients with significant pulmonary hypertension. There was no significant effect on mean arterial pressure or systemic vascular resistance at any time after inhalation in either group. Furthermore, there was no influence on extravascular lung water or intrathoracic blood volume. CONCLUSIONS: We conclude that inhaled aerosolized milrinone for a short period selectively dilates the pulmonary vasculature in heart transplant candidates with elevated pulmonary arterial pressure, without producing systemic side effects. Further comparative studies are necessary to evaluate possible advantages of milrinone compared to other inhaled vasodilators.     

Problems in assessment of pulmonary parenchymal consolidation in heart transplant candidates.

Radiologic assessment of the cause of pulmonary parenchymal consolidation in end-stage heart failure may be difficult. From August 1982 to May 1989, 22 patients being considered for orthotopic cardiac allografts had parenchymal consolidation on their chest radiographs, most commonly in the right lower lobe. Our purpose was to determine from standard radiologic studies whether this consolidation represented alveolar pulmonary edema in an atypical basal distribution, pneumonia, or pulmonary infarction. This differentiation is important because pneumonia is an absolute and infarction is a relative contraindication to surgery, whereas successful transplantation can be performed in a setting of pulmonary edema. The chest radiographs were reviewed retrospectively. When available, pulmonary angiograms, nuclear medicine ventilation/perfusion scans, and needle biopsy findings were also evaluated. The radiologic assessment was correlated with the results of surgical, autopsy, or clinical outcome. None of the conventional modalities was very accurate--the plain chest film was correct in only 63%, nuclear medicine studies in 50%. Angiography was the single most useful test, with an accuracy of 75%.     

Acinetobacter mediastinitis in a heart transplant patient

We report a 56-year-old who underwent orthotopic heart transplantation following left ventricular assist device bridge to transplantation. His postoperative course was complicated by severe sternal wound infection with Acinetobacter species requiring extensive debridement and reconstruction with a pectoralis major flap. This was followed by a total sternectomy, pectoralis flap debridement, and vacuum-assisted dressing.     

Esophageal cancer surgery in heart transplant patients

We herein report about 2 heart transplant patients undergoing surgical resection for esophageal cancer. Both were long-term survivors after orthotopic heart transplantation for dilated cardiomyopathy. One patient underwent a transthoracic esophagectomy and gastric pull-up for an early squamous cell carcinoma of the infracarinal esophagus. The second patient underwent a resection of the cervical esophagus and interposition of a free jejunal segment after neoadjuvant radiochemotherapy for a locally advanced squamous cell carcinoma in the cervical esophagus.     

Noninvasive estimation of pulmonary vascular resistance improves portopulmonary hypertension screening in liver transplant candidates

Despite limitations in sensitivity and specificity, estimation of the pulmonary artery systolic pressure (ePASP) on echocardiography is used for portopulmonary hypertension (PoPH) screening in liver transplant (LT) candidates. We proposed that alternative echocardiographic models, such as estimated pulmonary vascular resistance (ePVR), may provide improved testing characteristics in PoPH screening. In a retrospective analysis of 100 LT candidates, we found that the formula ePVR = ePASP/VTI_RVOT + 3 if MSN (VTI_RVOT = right ventricular outflow tract time velocity integral; MSN = mid‐systolic notching of the VTI_RVOT Doppler signal) significantly improves accuracy of PoPH screening compared to ePASP. We determined the optimal ePVR cutoff for PoPH screening to be 2.76 Wood units, as this cutoff provided 100% sensitivity and 73% specificity in screening for clinically significant PoPH. Comparatively, ePASP at a cutoff of 40 mm Hg provided 91% sensitivity and 48% specificity. We devised a new screening algorithm based on the use of ePVR at intermediate ePASP values (35‐54 mm Hg), and we confirmed the testing characteristics of this algorithm in a separate validation cohort of 50 LT candidates. In screening LT candidates for PoPH, the ePASP lacks accuracy, leading to unnecessary RHCs and undiagnosed cases of PoPH. A screening algorithm which incorporates the ePVR may be more reliable.     

Right ventricle-sparing heart transplant: promising new technique for recipients with pulmonary hypertension

Background. Right heart failure remains the leading early cause of mortality after heart transplantation, especially with antecedent pulmonary hypertension. Paradoxically, the discarded recipient right heart, acclimated to pulmonary hypertension, is often stronger than its nonconditioned donor replacement. Heterotopic (“piggy-back”) transplantation is plagued by problems related to the retained, dilated, hypocontractile left ventricle (lung compression, systemic emboli, arrhythmias). Were it possible to retain the recipient’s right heart, excising only the left ventricle, this could have important advantages, especially in severe pulmonary hypertension. This report describes such a technique.

Methods and Results. In four transplantation experiments (dogs), right ventricular-sparing transplantation proved technically feasible and hemodynamically successful. Bleeding after excision of the left ventricle was easily controlled. Back-bleeding from the native aortic valve (now open into the pericardial space) was not problematic. All atrial, aortic, and pulmonary arterial connections proved feasible. The preserved recipient right heart of all animals remained in stable sinus rhythm. All recipients were easily weaned from cardiopulmonary bypass, maintaining mean arterial pressures 60 to 110 mm Hg.

Conclusions. This investigation develops a technique for donor right ventricle sparing in cardiac transplantation, demonstrating technical and hemodynamic feasibility. This method holds promise for the unsolved clinical problem of right heart failure after orthotopic heart transplantation with antecedent pulmonary hypertension.     

Planning helps, behavioral disengagement does not: coping and depression in the spouses of heart transplant candidates

OBJECTIVE: To describe the coping strategies used by the spouses of cardiac patients who are pursuing heart transplant and to determine whether coping strategies predict depression in these spouses. METHOD: This is a cross-sectional design with 28 spouses (86% female) of cardiac patients who were being evaluated for heart transplant at a large medical center. Coping styles were measured using the COPE Inventory (Carver CS, Scheier MF, Weintraub JK, J Pers Soc Psychol 1989: 56: 267). Depression was assessed with the Center for Epidemiological Studies Depression Scale (Radloff LS, Appl Psychol Meas 1977: 1: 385). RESULTS: Spouses reported using adaptive coping strategies more than the less adaptive strategies. Increased levels of depression were positively correlated with 'behavioral disengagement' and negatively correlated with 'planning' coping strategies. CONCLUSIONS: Most studies find that maladaptive coping styles are associated with psychological distress. Consistent with these findings, we found that behavioral disengagement was associated with increased levels of depression. However, in the present study the more adaptive coping strategy of planning was associated with lower levels of psychological distress. These findings suggest that in the spouses of heart transplant candidates, both maladaptive and adaptive coping strategies may serve as markers of the presence or absence of emotional distress and thus may help in identifying spouses who may benefit from psychological, social work, or nursing interventions.     

Bicaval anastomosis in a heart transplant recipient with left superior vena cava

We describe the surgical procedure of orthotopic heart transplantation (OHT) in a recipient with persistent left superior vena cava (LSVC) and isolated noncompaction of the left ventricle. The bicaval anastomosis technique was performed using and isolating his native coronary sinus to let the left superior vena cava drain into his own inferior vena cava through the native coronary sinus.     

Survival of patients removed from the heart transplant waiting list

OBJECTIVE: End-stage heart failure has been associated with high mortality in the absence of transplantation. We evaluated the outcome of patients receiving optimal medical therapy who were removed from the cardiac transplant waiting list to determine survival and predictors of mortality. METHODS: We performed a retrospective review of 27 patients removed from the cardiac transplant waiting list from 1999 to 2001 at our institution. RESULTS: Mean age was 53 +/- 11 years; 16 of the patients were male. Status was IB in 3 cases and II in 24. Median time on the list was 32 months, and median follow-up was 2.9 years. Patients were removed from the transplant list because of either clinical improvement (group A, n = 18) or deterioration (group B, n = 9). In group A, 13 patients had improved functional status and 10 were in New York Heart Association class 1 or 2; 16 had improved echocardiographic left ventricular function. Survivals at 3 years were 100% in group A and 44% in group B (P <.01). CONCLUSION: Patients with end-stage heart failure who have clinical response to medical therapy have excellent 3-year survival. These data suggest the necessity of close evaluation of patients waiting for transplantation, with a low threshold for inactivation if persistent clinical improvement is observed.